Mutant protein in Huntington disease is resistant to proteolysis in affected brain (Q40774768)

23 September 2017

Mutant protein in Huntington disease is resistant to proteolysis in affected brain (English)

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23 September 2017

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2

C T McMurray

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23 September 2017

R B Dyer

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PubMed ID

11600884

23 September 2017

language of work or name

English

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publication date

1 November 2001

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23 September 2017

9

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inferred from page(s)

published in

Nature Genetics

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23 September 2017

29

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23 September 2017

3

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23 September 2017

270-278

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https://scigraph.springernature.com/pub.10.1038/ng745

23 September 2017

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cites work

Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract

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7 January 2021

Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

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7 January 2021

Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.

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7 January 2021

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

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7 January 2021

Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?

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7 January 2021

Nuclear inclusions in glutamine repeat disorders: are they pernicious, coincidental, or beneficial?

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7 January 2021

Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

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7 January 2021

Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum.

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7 January 2021

Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture.

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7 January 2021

The influence of huntingtin protein size on nuclear localization and cellular toxicity.

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7 January 2021

Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats

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7 January 2021

A cellular model that recapitulates major pathogenic steps of Huntington's disease

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7 January 2021

Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates

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7 January 2021

Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.

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7 January 2021

In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease.

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7 January 2021

Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition.

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7 January 2021

Caspase-8 is required for cell death induced by expanded polyglutamine repeats.

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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

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Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin

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Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease

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Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.

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Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells

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Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

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Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo

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Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

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Formic acid dissolves aggregates of an N-terminal huntingtin fragment containing an expanded polyglutamine tract: applying to quantification of protein components of the aggregates

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Differential loss of striatal projection neurons in Huntington disease

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Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease

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7 January 2021

CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain

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Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

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Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

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Huntington's disease intranuclear inclusions contain truncated, ubiquitinated huntingtin protein

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Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length

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Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

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Widespread expression of Huntington's disease gene (IT15) protein product

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The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

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Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

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A molecular investigation of true dominance in Huntington's disease

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Polyglutamine-mediated aggregation and cell death

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Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

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Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

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Trinucleotide expansion in haploid germ cells by gap repair

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Regulated expression of the prodynorphin gene in the R2C Leydig tumor cell line

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Neuropathological classification of Huntington's disease

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7 January 2021

Identifiers

DOI

10.1038/NG745

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PubMed ID

11600884

Dimensions Publication ID

23 September 2017

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PubMed ID

11600884